Typical vasculitic neuropathy commonly manifests as a subacute multiple mononeuropathy, symmetrical or asymmetrical sensori-motor polyneuropathy. Various clinical presentations of peripheral neuropathy may confuse the diagnostic approach sometimes. A 76-year old man presented progressive areflexic ascending paralysis. gait ataxia, severe vibration and position sense impairment for 1 month. We initially considered as subacute demyelinating polyneuropathy. Electrophysiologic studies showed mainly axonopathy with some evidence of demyelination. We performed sural nerve biopsy and diagnosed as vasculitic neuropathy. No laboratory data or clinical findings favored systemic vasculitic involvement. So we concluded vasculitis confined to the peripheral nerves. Vasculitic neuropathy rarely presented as large myelinated nerve fiber disease. It should be considered the clinical variability of vasculitic neuropathy so extensive studies are needed in the case of peripheral polyneuropathy with indefinite cause.
Aged ; Biopsy ; Demyelinating Diseases ; Gait Ataxia ; Humans ; Mononeuropathies ; Nerve Fibers, Myelinated ; Paralysis* ; Peripheral Nerves ; Peripheral Nervous System Diseases ; Polyneuropathies ; Proprioception ; Sural Nerve ; Vasculitis ; Vibration

We analyzed the practical problems and factors which affect making the correct differential diagnosis in the interpretation or test results from the nocturnal penile tumescence (NPT) test and erotic stimulation test (EST). This was done to provide better information for higher diagnostic accuracy in the clinical application of these tests. The followings are the results of NPT tests and EST identifying the factors affecting correct differential diagnosis by comparison the other differential diagnostic methods. The overall sensitivity of NPT test (Number: 114 total patients) was 82%. 21 cases (18%) could not be diagnosed correctly due to traction of the sensor (12 cases, 10%) and sleep disturbance (9 cases, 8%). The overall sensitivity of EST (Number: 174 total patients) without considering the degree of patient`s sexual drive to erotic stimulation was 77%. 40 cases (23%) could not be diagnosed correctly due to tolerance to pornographic film (17 cases, 10 %), discomfort by the body attachments (14 cases, 8%) and traction of the sensor (9 cases, 5%). However, higher sensitivity (90%) and lower rate of incorrect diagnosis (10% ) were observed in 119 patients who showed Grade II or III (moderate to good) sexual drive to erotic stimulation. The results suggest that undesirable factors in the primary screening methods, traction of sensor, sleep disturbance in NPT test, and tolerance to pornographic film, discomfort by the body attachments, traction of sensor in EST must be taken into consideration when interpretation of test results is being performed.
Diagnosis ; Diagnosis, Differential ; Diagnostic Tests, Routine* ; Erectile Dysfunction* ; Humans ; Male ; Mass Screening ; Penile Erection ; Traction

PURPOSE: Many pediatric urologists still favor using prophylactic antibiotics to treat children with vesicoureteral reflux (VUR). However, breakthrough infection sometimes occurs, leading to significant increases in morbidity as a result of renal scarring. Therefore, we tested whether abnormal renal scan and other factors are predictive of breakthrough infection using univariate analyses. MATERIALS AND METHODS: We retrospectively reviewed the medical records of 163 consecutive children who were diagnosed with vesicoureteral reflux between November 1997 and June 2010. Clinical parameters for the statistical analysis included form of presentation, gender, age, VUR grade, laterality, presence of intrarenal reflux, class of antibiotic drug, and presence of abnormal renal scan by Dimercapto-succinic acid. Clinical parameters used for prognostic factors were established by univariate analyses. Fisher's exact test and unpaired t-test were done using SPSS software [SPSS ver. 12.0 (SPSS Inc., Chicago, IL, USA)]. RESULTS: Breakthrough infection developed in 61 children (48.0%). A total of 58 children (45.7%) had abnormal renal scans. Time to development of breakthrough infection was significantly longer in girls (9.0+/-8.2 months) than in boys (5.8+/-4.8 months, p<0.05). On univariate analysis, though statistically not significant, the most predictive factor of breakthrough infection was abnormal renal scan (p=0.062). In patients with abnormal renal scans, breakthrough infection was not associated with mode of presentation, gender, grade or prophylactic antibiotics. However, there was a significant difference between patients younger than 1 year and those 1 year old or older. Mean+/-SD age at diagnosis of VUR in patients with breakthrough infection (1.14+/-3.14) was significantly younger than in those without breakthrough infection (5.05+/-3.31, p=0.009). There was also a significant difference between patients with bilateral or unilateral reflux (p=0.028). CONCLUSION: Our data showed that abnormal renal scan was the most predictive factor of breakthrough infection and demonstrated statistical significance in patients under the age of 1 year. Parents and physicians should remain aware that these patients are at high risk of breakthrough urinary tract infection, which may potentially lead to renal damage.
Anti-Bacterial Agents/*therapeutic use ; Female ; Humans ; Infant ; Male ; Retrospective Studies ; Urinary Tract Infections/*drug therapy/etiology ; Vesico-Ureteral Reflux/complications/*drug therapy/*microbiology

BACKGROUND: Assessment of left ventricular function with conventional 2-dimensional echocardiography (2D echo) remains largely qualitative and subjective because the manual tracing of endocardial borders is laborious and tedious. An automatic border detection (ABD) echo has been recently developed that permits real-time measurements of chamber areas and cardiac function. METHODS: To determine usefulness of ABD echo, left ventricular cross-sectional areas were automatically measured from the parasternal short-axis image in 25 cases including 9 cardiac patients, and compared with those by off-line analysis of the conventional 2D echo image. RESULTS: In on-line ABD analysis, short axis end-diastolic area averaged 13.1+/-2.2cm2, end-systolic area 5.3+/-1.3cm2 and fractional area change 59.4+/-9.0% and off-line manual analysis yielded corresponding values of 14.7+/-2.5cm2, 6.2+/-1.3cm2, and 57.1+/-7.1%. The end-diastolic and end-systolic areas by both methods were significantly different, respectively. Left ventricular end-diastolic and end-systolic area and fractional area change obtained from ABD echo correlated significantly with those of off-line measurements(r=0.897, p<0.001 ; r=0.505, p<0.01 ; r=0.427, p<0.05). CONCLUSION: Thus, these facts suggest that ABD echo is useful for on-line continuous measurement of chamber areas and cardiac function.
Axis, Cervical Vertebra ; Echocardiography* ; Humans ; Ventricular Function, Left

A case of renal amyloidosis with crescentic glomerulonephritis associated with rheumatoid arthritis is described. A 60-year-old female with 15 years' history of rheumatoid arthritis developed nephrotic syndrome followed by rapid deterioration of renal function. Glomerular amyloid deposition and sclerotic change was present in kidney biopsy specimen and crescentic change was found in 85% of the glomerulus. Electron microscopic finding of glomerulus showed randomly oriented, rigid-appearing, long nonbranching fibrils. The patient was treated with intravenous high-dose methylprednisolone pulses combined with intravenous cyclophosphamide followed by oral corticosteroids. Three months after the above treatment, renal function gradually improved, reaching serum creatinine level to 2.5 mg/dL. But the nephrotic range proteinuria persisted.
Adrenal Cortex Hormones ; Amyloidosis* ; Arthritis, Rheumatoid* ; Biopsy ; Creatinine ; Cyclophosphamide ; Female ; Glomerulonephritis* ; Humans ; Kidney ; Methylprednisolone ; Middle Aged ; Nephrotic Syndrome ; Plaque, Amyloid ; Proteinuria

Exploding head syndrome (EHS) is characterized by a sudden loud imagined noise or sense of a violent explosion in the head occurring as the patient is falling asleep or waking during the night. EHS is classified as "other parasomnia" on the ICSD-2 (International Classification of Sleep Disorders), and the neurophysiologic mechanisms underlying this hypnagogic phenomenon are unknown. EHS is a rare type of other parasomnia that has not been reported in Korea. We report two cases of EHS that presented with typical clinical symptoms.
Explosions ; Head ; Humans ; Korea ; Noise ; Parasomnias

BACKGROUND: Proton beam radiotherapy (PBT) has shown to provide high radiation dose to tumors and to save surrounding normal tissues because of its physical characteristics, Bragg peak. In the current study, we report the early outcomes for pediatric patients with intracranial gliomas treated with PBT and compared PBT plan (pencil beam scanning and double scattering) with intensity modulated radiotherapy (IMRT) plan and three dimensional-conformal radiotherapy (3D-CRT) plan.METHODS: Clinical data from 18 consecutive children with intracranial gliomas who underwent PBT from May 2007 to April 2012 was collected. The median follow-up duration was 16 months (range 6-69).RESULTS: There were 9 patients with brain stem glioma, 2 patients with optic pathway glioma, 2 patients with low grade glioma (LGG), 2 patients with anaplastic astrocytoma (AA) and 3 patients with glioblastoma multiforme (GBM). The median overall survival for patients with brain stem glioma was 11 months. Patients with optic pathway glioma, LGG or AA were all alive without progression except one patient. Among patients with GBM, one patient had no evidence of disease 25 months after PBT. When PBT plan was compared to those of IMRT and 3D-CRT for patients with LGG or AA and one patient with brain stem glioma by DVH analysis, PBT showed better sparing effect on normal tissue compared to IMRT and 3D-CRT, especially in low dose area.CONCLUSION: PBT could be delivered safely and effectively to pediatric patients with gliomas. For confirming the clinical benefits of PBT, further follow-up is necessary.
Astrocytoma ; Brain Stem ; Child ; Follow-Up Studies ; Glioblastoma ; Glioma ; Humans ; Organ Sparing Treatments ; Pediatrics ; Proton Therapy ; Protons

PURPOSE: Endometriosis is a condition where uterine mucosal tissue is located outside the uterus, and may be pelvic or extrapelvic. Extrapelvic endometriosis is a diagnostic challenge due to its very low incidence. The aim of this study was to analyze the clinical features of extrapelvic endometriosis in order to help diagnosis and treat this condition. METHODS: Between January 1998 and May 2005, 6 patients diagnosed with extrapelvic endometriosis after surgery at our hospital were retrospectively reviewed by a telephone interview. RESULTS: All cases were women in their reproductive years, with a median age of 37.5 years (range 34~50 years). Five of the six cases had a prior medical history of a caesarean section and 1 case had episiotomy during a normal vaginal delivery. Five cases were located in the lower abdomen and 1 case was located in the perineum. All patients presented with a surgical scar mass associated with pain and size that increased during menstruation. The median mass size was 2.5 cm (range 1~3.4 cm). The mass was found after a median interval of 13 months (range 1~45 months) from pelvic surgery. All cases were suspected of having extrapelvic endometriosis due to their specific clinical features, and 2 of them were confirmed by fine needle aspiration cytology (FNAC). All the patients were treated with a surgical excision. There was no recurrence after a median follow up of 27 months (range 4~86 months). CONCLUSION: Patients with a surgical scar mass associated with pain that increases in size during menstruation should be suspected of having extrapelvic endometriosis and be treated by a complete surgical excision to prevent a recurrence.
Abdomen ; Biopsy, Fine-Needle ; Cesarean Section ; Cicatrix ; Diagnosis ; Endometriosis* ; Episiotomy ; Female ; Follow-Up Studies ; Humans ; Incidence ; Interviews as Topic ; Menstruation ; Mucous Membrane ; Perineum ; Pregnancy ; Recurrence ; Retrospective Studies ; Uterus

Synovial sarcoma occurring in the pleura and lung is extremely rare. We report a case of pleuropulmonary synovial sarcoma as a second malignant neoplasm. The patient had been diagnosed with acute myelomonocytic leukemia at 5 years of age, and received matched sibling donor allogeneic bone marrow transplantation, with total body irradiation and cyclophosphamide conditioning. At 22 years of age, he complained of worsening chest discomfort and exertional dyspnea. Chest CT revealed a huge mass in the right middle lobe, pleura, and diaphragm. The patient was initially diagnosed as sarcomatoid malignant mesothelioma, without any environmental or occupational asbestos exposure. Five months later, the patient presented with soft tissue metastasis and underwent needle biopsy. Pathological examination including SYT-SSX RT-PCR revealed synovial sarcoma, which led to a review of the original tumor findings and confirmed the diagnosis of pleuropulmonary synovial sarcoma.To the best of our knowledge, our patient is the first case of pleuropulmonary synovial sarcoma developed after allogeneic hematopoietic stem cell transplantation.

Highly pathogenic avian influenza (HPAI) viruses have caused severe respiratory disease and death in poultry and human beings. Although most of the avian influenza viruses (AIVs) are of low pathogenicity and cause mild infections in birds, some subtypes including hemagglutinin H5 and H7 subtype cause HPAI. Therefore, sensitive and accurate subtyping of AIV is important to prepare and prevent for the spread of HPAI. Next-generation sequencing (NGS) can analyze the full-length sequence information of entire AIV genome at once, so this technology is becoming a more common in detecting AIVs and predicting subtypes. However, an analysis pipeline of NGS-based AIV sequencing data, including AIV subtyping, has not yet been established. Here, in order to support the pre-processing of NGS data and its interpretation, we developed a user-friendly tool, named prediction of avian influenza virus subtype (PAIVS). PAIVS has multiple functions that support the pre-processing of NGS data, reference-guided AIV subtyping, de novo assembly, variant calling and identifying the closest full-length sequences by BLAST, and provide the graphical summary to the end users.